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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
6
pubmed:dateCreated
2000-2-4
pubmed:abstractText
The association of diffuse bronchiolitis in patients with Kartagener's syndrome (KS) has not been reported previously. The aim of this study was to present the morphological characteristics of bronchiolitis in patients with KS. Eight patients (four males, four females; mean age 37.9+/-18.7 yrs), clinically diagnosed as KS with the classical triad of chronic pansinusitis, bronchiectasis and situs in versus with dextrocardia, were evaluated. Routine chest radiography showed bronchiectasis and dextrocardia in all patients. Chest computed tomography (CT) showed diffuse centrilobular small nodules up to 2 mm in diameter throughout both lungs in six out of eight patients. Pulmonary function tests revealed marked obstructive impairment in all patients (forced expiratory volume in one second 57.0+/-11.3%, residual volume/total lung capacity 45.+/-12.7%, maximum midexpiratory flow 0.92+/-0.72 L x s(-1), forced vital capacity 74.1+/-12.2% (all mean +/- SD)). The examination of cilial movement of the bronchus revealed immotility in all of the five patients examined. The ultrastructure showed ciliary dynein arm defects in all patients. Histopathological examination of lung specimens obtained at autopsy or by video-assisted thoracoscopic surgery showed obliterative thickening of the walls of the membranous bronchioli with infiltration of lymphocytes, plasma cells and neutrophils, but most of the distal respiratory bronchioli were spared and alveolar spaces were overinflated. Pathologically, the diffuse centrilobular small nodules on the chest CT mainly corresponded to membranous bronchiolitis. This is the first report demonstrating that the association of diffuse bronchiolitis might be one of the characteristic features of the lung in Kartagener's syndrome.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Dec
pubmed:issn
0903-1936
pubmed:author
pubmed:issnType
Print
pubmed:volume
14
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1332-9
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed-meshheading:10624763-Adolescent, pubmed-meshheading:10624763-Adult, pubmed-meshheading:10624763-Aged, pubmed-meshheading:10624763-Anti-Bacterial Agents, pubmed-meshheading:10624763-Bacterial Infections, pubmed-meshheading:10624763-Biopsy, pubmed-meshheading:10624763-Bronchiolitis, pubmed-meshheading:10624763-Diagnosis, Differential, pubmed-meshheading:10624763-Drug Therapy, Combination, pubmed-meshheading:10624763-Female, pubmed-meshheading:10624763-Humans, pubmed-meshheading:10624763-Kartagener Syndrome, pubmed-meshheading:10624763-Lung, pubmed-meshheading:10624763-Male, pubmed-meshheading:10624763-Middle Aged, pubmed-meshheading:10624763-Prognosis, pubmed-meshheading:10624763-Respiratory Function Tests, pubmed-meshheading:10624763-Sputum, pubmed-meshheading:10624763-Thoracoscopy, pubmed-meshheading:10624763-Tomography, X-Ray Computed, pubmed-meshheading:10624763-Treatment Outcome
pubmed:year
1999
pubmed:articleTitle
Bronchiolitis in Kartagener's syndrome.
pubmed:affiliation
Division of Respiratory Diseases, Toranomon Hospital and Okinaka Memorial Institute for Medical Research, Tokyo, Japan.
pubmed:publicationType
Journal Article, Case Reports, Research Support, Non-U.S. Gov't